Journal of the Louisiana State Medical Society
Figure 2: (A) Skin punch biopsy showing a superficial and deep dermal predominantly perivascular infiltrate of tumor cells (H&E 20x). (B) Skin biopsy showing dense infiltrate of tumor cells with irregular nuclei, fine chromatin, and prominent nucleoli (H&E 500x under oil immersion). (C-D) Tumor cells showing positivity for CD4 (C) and CD56 (D) (IHC 500x under oil immersion).
volved by the same tumor cells. Fine needle aspiration of subcarinal lymph node showed infiltrate of the tumor cells. CSF showed detection of CD4+CD56+ tumor cells compris- ing 32%of CD45-positive cells by flow cytometry. Peripheral blood smears showed circulating blasts. Cytogenetic analy- sis showed normal male karyotype 46, XY. 20 FISH analysis showed no translocation affecting TCR A/D constant re- gion. Gene rearrangement studies of TCR gamma and JH of IgH were negative. Serological studies showed that the patient was positive for EBVVCA IgG (3.30; reference range 0.00-1.09) and negative for EBV VCA IgM (0.13; reference range 0.00-1.09). The patient was diagnosed with BPDCN and treated with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy for six cycles, as well as intrathecal therapy. The patient achieved only partial remission and subsequently was treated with ICE (ifosfamide, carboplatin, and etoposide) chemotherapy for two cycles. While waiting for bone marrow transplantation
(BMT), the patient had a relapse of the disease with newly developed multiple 1-1.5 cm slightly raised red lesions on the extremities in August 2010. Skin punch biopsy showed a superficial and deep dermal predominantly perivascular infiltrate of tumor cells with irregular nuclei, fine chromatin, and prominent nucleoli. By IHC, the tumor cells were posi- tive for CD4 and CD56 but negative for CD3. The patient was treatedwith BEAM (carmustine, etoposide, cytarabine, and melphalan) chemotherapy, along with targeted radia- tion for the skin lesions, but showed only partial response. The patient then receivedmatched related donor allogeneic peripheral blood stem cell transplantation (SCT) in August 2010. In January 2011, the patient had another relapse of the disease with new skin lesions. By IHC, skin punch bi- opsy showed the tumor cells were positive for CD4, CD43, CD56, and CD123 and negative for CD3, CD5, CD8, CD20, CD57, CD68, and TDT (Figure 2A-2D). EBER by in situ hybridization was negative. Complete blood count showed
4 J La State Med Soc VOL 166 January/February 2014
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